The Primary Immunodeficiency Working Party (PID WP) with the Czech society for Allergy and Clinical Immunology (ČSAKI) was established in 2014. The main reason for its creation was the specific need for collaborative effort and specialist care for patients with congenital immunodeficiencies. The historically problematic and systematically unresolved immunoglobulin substitution therapy represents another area of interest, thus establishing clear rules for immunoglobulin therapy was one of the founding tasks and goals of the Working Party.
The Working Party is open to any healthcare practicioner or researcher interested in primary immunodeficiencies. The Member list lists all the current members and is updated regularly.
While the activity of the Working Party encompasses the problematic of PIDs in general, the specific topics and tasks are the following:
Czech PID Registry
The national PID patient registry is now well established and administered by the Department of Clinical Immunology and Allergy at St. Anna’s University Hospital, Brno. The registry’s current status can be explored at its site (Czech PID registry) and is update regularly. It’s a tool for mapping PIDs within the Czech Republic, but also serves as a foundation for negotiations with health insurance providers, the Czech Ministry of Healthcare and others involved in financial aspects of care given to patients with PID diagnosis.
Most Czech PID patients are also reported into the ESID registry, administered by ESID (European Society for Immunodeficiencies). Thanks to this, Czech patients, research and caregiver groups are included in international publications based on the registry data.
PID diagnostic tools and strategy
The long-term aim of PID Working Party is to unify and simplify diagnostic process of primary immunodeficiencies. Currently, a high level standard of diagnostic practice is in place in the Czech Republic, owing to well-established cooperation amongst existing centers. The principal immunologic tests are widely available, more specialized tests (especially genetic testing) are to certain degree restricted to central facilities, but are generally accessible.
Cooperation within the PID Working Party allows for effective and advanced approach to each and every PID patient in the Czech Republic.
Newly emerging New Generation Sequencing methods have been successfully implemented on research grounds, already directly impacting clinical practices. Wider incorporation of the method into diagnostic algorithms is expected soon, althouth initially limited to national centers in Prague and Brno.
Since 2013 TREC/KREC testing has been available, providing an excellent tool for early detection of most severe forms of PIDs. The tests, as well as the interpretations, are carried out in national centers in Prague and Brno. PID Working Party members actively participate in debates concerning use of this test for national neonatal PID screening.
More information about diagnostic tools may be found on Available Tests page.
Primary Immunodeficiencies Treatment
Treatment of patients with PID is provided countrywide in accordance with currently available guidelines. The principal tool is immunoglobulin therapy, which is regrettably restricted by its financial aspects. Centralized treatment strategy for PID patients hasn’t been established yet (see also register of meetings between Ministry of Healthcare and health insurance providers). The need for rational budgeting under economic restrictions inflicted on centers providing immunoglobulin therapy is reflected in under dosing of PID patients. Ensuring the availability of immunoglobulin therapy is a subject of continuous discussions amongst relevant parties. Generally, the availability of intravenous and subcutaneous immunoglobulins in the Czech Republic is very good.
Hematopoietic stem cell transplant availability and results in the Czech Republic are equivalent to foreign leading transplant institutions. The current operations of one national transplant center appear to be fully sufficient and require no further extension.
Primary immunodeficiencies research activities
National PID centers, as well as external collaborators participate in extensive range of research activities. PID Working Party aim is to monitor the ongoing research projects and related publications. Further goal is to expand involvement of our junior colleagues in this field.
Complete list of PID related publications of PID Working Party members since 2015 may be found on page Publications.
Selected PID related full texts are available on page Materials.
International cooperation
Majority of PID Working Party members are also members of ESID, European Society for Immunodeficiencies, and actively participate in ESID events. Junior colleagues are members of ESID Juniors and ESID Junior Country Representatives groups. Regular meetings and international workshops are held in the Czech Republic under ESID patronage, organized by PID Working Party members, presided by professor Anna Šedivá.
Further information on ESID related activities may be found on ESID page.
Primary immunodeficiencies center in University Hospital Motol is also Jeffrey Modell Foundation center, an American non-profit organization focusing on public education and promoting knowledge about primary immunodeficiencies.
Regional meetings (see list of scheduled events) are also essential for the PID Working Party operation. Flow cytometry methods for screening and diagnosing primary immunodeficiencies are being developed in laboratories in Prague and Brno as part of EuroFlow PID group. Intense international cooperation takes place within specialized groups and is reflected in publishing activities. Some PID Working Party members are also members of Inborn Errors Working Party (IEWP) of European Society for Blood and Marrow Transplantation.